皮肤淋巴瘤的现状与展望

Current and future aspects of cutaneous lymphomas

皮肤淋巴瘤是指原发于皮肤的具明显异质性的淋巴增殖性疾病。病因及发病机制不明，可能为一种多因子作用于具有遗传缺陷的淋巴细胞诱发的累加效应和逐渐发展的过程。皮肤淋巴瘤的分类和分期需以阐明组织起源、判定生物行为并指导临床治疗为目的，经过不断完善后，现已形成最新的WHO／EORTC分类系统。尽管分子病理学对某些皮肤淋巴瘤的早期诊断和生物行为判断具有重要价值，但皮肤淋巴瘤的确定诊断，还需要结合临床、组织病理、免疫表型和分子特征等的综合资料。皮肤淋巴瘤的标准化治疗方案可能难以统一，治疗方法的选择需以分期为基础，各种新的实验性生物学治疗方法正不断应用于临床。

Cutaneous lymphoma （CL） is a heterogeneous lymphoproliferative entity that primarily occurs in skin. Although the etiology and pathogenesis of CL remain unknown, there is increasing evidence that the carcinogenesis is a multifactorial stepwise evolutionary process arising from the accumulation of genotraumatic lymphocytes. The system of CL should aim to define the origination and biological behaviors as well as to guide the clinical treatment of these tumors. A new WHO/EORTC classification system has been established for CL. Molecular pathology can provide important information for early diagnosis and on biological behavior of CL, however, the final diagnosis should be based upon a constellation of clinical, histopathological and molecular findings. It may be difficult to make a standard regimen for the treatment of CL, a stage-adapted therapy should be recommended. Fortunately, varied experimental biological modalities have been continuously used for its treatment.